Paroxysmal nocturnal haemoglobinuria

 

Alternative names, keywords

PNH

Samples required

EDTA blood (purple cap/ black cap ring, 4 mL tube). Smaller tubes are available for paediatric samples.

Samples are stable for <48 hours. Not tested at weekends or Bank Holidays so samples must arrive at RSCH by 3pm on Fridays.

Test indications

Requests through a Consultant Haematologist only.

Consideration for testing for PNH should be made in the following circumstances:

  • Patients with haemoglobinuria
  • Patients with Coombs-negative intravascular haemolysis (based on abnormally high serum LDH), especially patients with concurrent iron deficiency
  • Patients with venous thrombosis involving unusual sites*
    • Budd-Chiari syndrome
    • Other intra-abdominal sites (e.g. mesenteric or portal veins)
    • Cerebral veins
    • Dermal veins
  • Patients with aplastic anaemia (screen at diagnosis and once yearly even in the absence of evidence of intravascular haemolysis)
  • Patients with refractory anemia-MDS
  • Patients with episodic dysphagia or abdominal pain with evidence of intravascular haemolysis

Source: Diagnosis and management of paroxysmal nocturnal hemoglobinuria, Parker. C., et al, Blood 106(12), 2005

Reference range

Reference ranges not applicable - normal patients should have 0% PNH clones in both red cells and white cell populations.

Turnaround time

Turnaround time 7 days (not tested at weekends or Bank Holidays).

Enquiries

Haematology (Immunohaematology)

 

Details